RECORDED Wed 20 Nov 2024
SUMMARY KEYWORDS
hereditary angioedema, modern therapies, spontaneous mutation, blood protein defect, normal life, stress triggers, invisible illness, genetic therapy, delayed diagnosis, mental health, sibling impact, school challenges, medication access, rare disease, advocacy work
SPEAKERS
Fiona Wardman, Megan Gilmour
Megan Gilmour 00:00
This episode is proudly brought to you by the TPG Telecom Foundation dedicated to creating opportunities to improve the health, wellbeing and education of Australian communities in need. Hi, I’m Megan Gilmour, MissingSchool, co-founder and CEO, and this is Live & Learn.
This is the podcast that unearths insights at the intersection of health and education, building a powerful alliance to keep kids with complex health conditions connected to school from anywhere. The podcast is recorded on Ngunnawal land, and we acknowledge and pay respect to the traditional owners of this land and the land on which you’re listening.
Welcome to our weekly webcast, a place to zero in on the interplay between health and education and to amplify the voices of those supporting school students to navigate their educational journeys despite complex health challenges.
Today on Live & Learn, we have Fiona Wardman. Fiona Wardman lives with hereditary angioedema or HAE and is executive VP of global advocacy and Chief Diversity Officer for HAE International, a global nonprofit network dedicated to raising awareness and improving the lives of people living with HAE.
Fiona has been a board member since 2014, and she also serves as treasurer and regional patient advocate for Oceania and South Africa. Fiona co-founded the Australasian HAE organisation in 2011 and has held leadership roles in various industries.
Welcome, Fiona. We’re so excited to have you on Live & Learn.
Fiona Wardman 01:49
Thank you for the invitation.
Megan Gilmour 01:51
Our absolute pleasure. So, Fiona, today we’re here to talk about supporting school connections for students with complex medical or mental health conditions. Let’s dive right on into the questions.
Fiona tells us how hereditary angioedema impacts kids and why it’s something every Australian needs to know and care about.
Fiona Wardman 02:16
Hereditary angioedema, or HAE, is a disease that affects the protein in the blood of the patients that have it. It causes recurrent swelling, episodes which can be very spontaneous, can be brought about by trauma, stress, hormones, or no reason at all, and these episodes present with swelling, which can be external in any part of the body, and also internally in the stomach, which is excruciatingly painful, also if it affects the throat, the person with HAE can suffocate and die, unfortunately.
Megan Gilmour 03:01
Wow, that is something that every Australian needs to know and care about, right there. Now, many people probably assume things about HAE. What’s the million-dollar question you get from parents or loved ones when their child is diagnosed with or experiences HAE?
Fiona Wardman 03:22
I think the biggest question is, can they live a normal life? And the answer to that is yes.
So, I might just go back one step and talk about hereditary angioedema and how that is passed down from generation to generation. One of the parents will need the defective gene in order to pass it on to their child. There is a 50% chance that that does happen.
However, there are 25% of us, like me, who weren’t passed down the defective gene. I was a spontaneous mutation, so I went through all of my schooling having these episodes and not having any explanation about what they were for. But yes, I’ve been able to have a normal life, and thanks generally to effective medications,
Megan Gilmour 04:17
Right, that’s just fascinating all on its own, I could dig right in there. I won’t, because I think we’re going to pick some of these questions up that I have as we as we roll through.
What’s the most important thing then that you want them to know?
Fiona Wardman 04:40
With a diagnosis, now there is the possibility of everybody with this disease having access to modern therapies and living a normal life. So, some of those modern therapies at the moment are injections – they may be twice a week, they may be once a fortnight, once a month. There are tablets coming our way.
Our rare disease has so many new treatments coming our way, which is incredible for a rare disease within a scope of seven to 8000 rare diseases, we have so much interest in our area.
And so, with these modern medications, children can get on and get on with their life and not have to worry about the stress of exam time, not have to worry about the stress of missing social functions, the bullying that comes along with that, the mental health issues that come along with that. So, living a normal life just like their peers and friends.
Megan Gilmour 05:39
Yeah, great. So, you’ve already addressed this, but I’ll ask it anyway. Fiona, we’d love to know what brought you into the world of HAE.
Fiona Wardman 05:56
Yes, so as I mentioned previously, I am also a patient with HAE. My symptoms started at the age of seven, and once or twice a year, I used to have a hand and arm swelling, which my mum would put into a sling so that I could go to school. It was painful a lot of the time because it was quite swollen and hot and quite heavy.
Sometimes the skin split, and just getting dressed or brushing your teeth or whatever is one of those things that you just can’t do with swollen limbs. So, I finally got diagnosed at the age of 33, so there’s 26 years that took for me to get a diagnosis, and there was a whole complication in between there of the different physicians that I went to and specialists to get a diagnosis, nobody knew what it was, but that was also in the time of no internet. So, that’s telling you how old I am.
So, but when I finally did get a diagnosis, I was asked by my physician to get involved with advocating for other patients and advocating for modern therapies so that we could have a normal life and not have to worry about these swelling episodes that come on at any time.
Megan Gilmour 07:24
Yes, so that was my little drill down question, is you said you had the spontaneous mutation, but it also sounds like this edema can happen spontaneously as well. Are there triggers?
Fiona Wardman 07:40
Yes, so for me, my biggest triggers were, or still are, hormones. Females, when they get to puberty seem to have a lot more attacks. Stress – and that’s good and bad stress, so exam time or any type of stress you know, being excited about going to a party, or, you know, a school camp or something like that, would bring on an attack. So, or if you sort of hit your arm or hit your foot, or whatever happened to be, if you were wrestling with somebody and something was, you know, a little bit too hard on the body, contact, that would start an attack.
Megan Gilmour 08:25
So, we’re talking about fluid in the tissues. Is that what it is?
Fiona Wardman 08:32
Yes. So basically, we don’t have enough of the complex protein, or we have abnormal protein in our blood system to not turn off any of the fluid that leaks through the blood vessels – so, like a bruise, for instance. So, in similar terms, people with hemophilia have a same sort of an issue, but ours is a different complex protein that we have that is defective, in one way or another, whether it’s not enough or it’s, we have enough, but they’re nonfunctional.
Megan Gilmour 09:12
Yes, has that got something to do with albumin?
Fiona Wardman 09:17
No. [both laughing].
I’m not a scientist, so that I can give you, you know, a lot on the higher level, drilling down to there is not something that you should be talking to me about.
Megan Gilmour 09:30
Yes, [laughter], you can tell that I’ve had some my own background in this, and remembering that in that sort of situation, in my son’s case, which, of course, wasn’t hereditary, but these things stick in our minds. Thank you for that.
Knowing all that you know then, what’s your biggest hope for kids with HAE, their families and loved ones when navigating this rare disease?
Fiona Wardman 10:06
I think the biggest hope is that there are treatment options for prevention of any of the attacks for every single person, not only in Australia and New Zealand who we look after, but around the globe as well.
And we’re quite lucky in Australia that we do have access to modern therapies. We don’t have the full suite that are out there, but that’s something we do with the government. We advocate to the government about the different treatments that are available. We do get access to clinical trials.
One exciting thing is genetic therapy. So, there is gene therapy where they inject the defective gene and correct it or cut it out, and then people don’t have the issues anymore. However, at this point, this is just for that person. If the affected person with HAE does have children, there is still the 50% chance, so it only fixes it in that one person. There are clinical trials going on with that at the moment, and they’ve been really successful.
Megan Gilmour 11:11
Well, that’s exciting news. There’s hope on the horizon there.
So, you mentioned misconceptions. What are some of the common and perhaps frustrating public misconceptions about HAE, and how have you seen this impact families?
Fiona Wardman 11:31
Okay, so with misconceptions you know, our disease does present like somebody’s been stung by a bee or a wasp or allergic to something. And so there is the misconception with emergency department staff or even specialists and doctors in general, that they automatically go towards an allergy diagnosis rather than – knowing the full extent of what they can do with a blood test to be able to diagnose HAE.
Now, as I said before, this disease is sort of one of 7000 to 8000 and so we don’t expect everybody to know everything about it, but our hope is that, in the physician’s side of things, that they’re starting to think a little bit more about the disease, and so therefore, getting the right blood tests sorted for the individual.
But also, in the larger community that you know, we have to wrap these children up in cotton wool. We don’t need to; yes, the stomach attacks are excruciatingly painful, and the throat attacks obviously are very life, they are life threatening, so but a hand or an arm or foot or whatever, we can still get on with our life.
And the other thing is that people look at you and when you say you’ve got a rare disease, and they say, well, you don’t look sick, because most days, I’m okay. But there’s only individual days or times during the month, for instance, where the attacks will come on, and that’s due to hormonal issues or whatever.
But we’re completely normal looking until we have an attack, and then that’s when the disease comes out. So, we’ve had people go to the hospital and there’s an internal stomach attack or something, and the physicians will say, ‘oh, there’s nothing wrong with you – I can’t see anything’, you know, but there clearly is. So, listening to the patients too, and listening to people with this disease, to help understand what they’re going through, and to know that basically we have great days every day. It’s just periods of time where this does come on – that’s when you’ll see it.
Megan Gilmour 14:04
Yes, so important to recognise there the nature of invisible illness and some of the flow on effects of that. And you’ve mentioned a few there. I’m sure there’ll be more that come up as we delve a little bit deeper here.
And the critical point about moving beyond the first line assessment of people presenting where it can look like an allergy, but not taking those further steps where that is obviously continuing to occur, and in order to get the right diagnosis.
How many Australian children and youth are living with HAE right now?
Fiona Wardman 14:50
I would estimate there’s around 100 based on our population and the population of the children in our country. So, there’s roughly 100 and we may know about 40 of those within our member organisation, our patient support group. They’re not all connected directly. It’s usually their parents that connect with us to get support and resources and access to meetings and so forth, and webinars that we do for more information and information about clinical trials and medications.
But there, there’s roughly about 100 I would say that do have it. The prevalence of this disease is one in 30,000 to one in 50,000 so potentially there’s 500 patients with HAE at the moment.
Megan Gilmour 15:46
In Australia?
Fiona Wardman 15:46
Yes.
Megan Gilmour 15:47
Yes. While it is rare, it is something all Australians need to know and care about. I’ll just add that again,
Fiona Wardman 15:56
Yes. Definitely
Megan Gilmour 15:58
And especially when we get to talking about school.
We’ve touched on this, please add anything else that you’d like to. Is there a risk of delayed diagnosis, or are most cases picked up easily?
Fiona Wardman 16:14
Generally, if there is HAE already in the family and it’s been diagnosed, then usually the parents will know to get their children tested from one year of age and onwards. Some people do wait until they have their first attack – the children have the first attack – which isn’t probably the best scenario, only because, you know, knowledge is power, so you know what to do and it also discounts the child having HAE if you get them diagnosed early and they don’t have it, so you can just get on with it and not have to worry about it.
And the other thing, obviously, is, like me, it took a very long time for a diagnosis because there was no family history, so making sure that the child, or the person presenting with the different symptoms that HAE brings, is sent to the right specialist, which would be an immunologist in our case, in Australia. And then a blood test would get done, and then the child would – or whoever, the adult even, I know this is for kids, but it took a long time for me to be diagnosed. So, it’s making sure that you’re on it.
So, if a child is presenting with recurrent swelling from some sort of stress or some sort of trauma bump or so forth, or for no reason at all, and the swelling takes four or five days to go up and down. That is not an allergy. If antihistamines don’t work, corticosteroids don’t work, adrenaline doesn’t work, then it’s probably HAE, and you should seek out the assistance of a referral to an immunologist to get the correct blood tests.
Megan Gilmour 18:07
Right. Great advice there. And does this mean due to the hereditary nature of it, unless there’s that spontaneous gene mutation, that more than one child in a family could have HAE and have you come across that?
Fiona Wardman 18:23
Yes. So, we have families that are under our umbrella, and they have a long family history of HAE, aunts, uncles, grandparents, whatever, parents and siblings, and then they have children, and their children don’t carry the gene. They are completely fine, HAE free.
And then you will have some that lose the lucky dip and have all of their children having HAE. And sometimes it’s half and half, so it’s a 50% chance of passing this disease onto each and every child, and it’s luck of the draw.
Megan Gilmour 19:02
Right. And just touching back lightly on your own experience. How did it feel when you finally got that correct diagnosis?
Fiona Wardman 19:16
Yes, that was like a weight was off my shoulders, because I had gone through all of those years thinking that I was, you know, strange. I actually had one physician tell me that I was just weird.
Megan Gilmour 19:30
Nice!
Fiona Wardman 19:16
And that’s not something that you want to hear, right? You want to go to physicians for some assistance, not to be told that you’re weird.
So, when I finally got a diagnosis, and the weight had been lifted off my shoulders, I was then told that there were other people just like me, which was also amazing, and I was able to speak to others pretty much straight away. And in fact, I met face to face with many patients about two years after my diagnosis, and that’s when we formed HAE Australasia.
So being in a room with people who I didn’t have to explain myself to, and who knew just exactly what I had been through all of my life was the most empowering situation I could have ever been in, and this is my hope with the advocacy work that we do within the organisation is to help people understand that there are people just like them, and to talk about it.
This is the other thing with this disease, is that it’s not a one size fits all. So, you can have two people in the one family with HAE, one will hardly get attacks, and one will be severe and quite often, and then we all handle things differently.
So, speaking to different people outside of the family is really amazing, because you get a different perspective, you get a different outlook, you get different tips and tricks, and you know that you’re not alone.
Megan Gilmour 21:00
This is a really important one, isn’t it? And I know through these interviews, this is something that I’m hearing all the time, it’s across the board, and I know it from my own experience too.
It’s almost like when you meet somebody with shared, lived experience, you almost don’t have to talk about the thing or explain it, and so you can just actually get on – feeling understood – you can just get on with being yourself, which is really such an important theme that’s coming through on these interviews, as well as that normalcy and feeling, being able to feel who you are as a person, not just be identified with the disease.
Fiona Wardman 21:34
Yes, exactly, and I have to just mention here that with the help of modern therapies – because we’ve had some older therapies which are no longer available in Australia, thankfully, however, they had their place at the time – and now we’ve got access to modern therapies designed for hereditary angioedema.
And living a normal life with those and being able to have access to those, I forget that I’m a patient, because now I don’t have any of those attacks or the symptoms, none of it.
And while I live and breathe being an advocate for this disease, I sometimes have to draw on the fact that I’m also a patient because it’s been filed in the back of my mind, because I don’t wake up every morning and go, am I going to have an attack today? Is it going to be bad? Where is it going to be? Is it going to stop me from doing this? Is it going to stop me from going there? Do I have to have another pair of shoes that are a size bigger so that my foot can fit in there? You know? Can I wear what I want to wear? Do I have to organise for somebody to take care of me? All of those things have been wiped away with the access to the therapies that aren’t totally designed for this disease.
Megan Gilmour 23:11
Yes, and just so much relief right there.
Fiona Wardman 23:15
Yeah, yeah.
Megan Gilmour 23:16
And this fits with my next question, which is, what kind of complication do kids with HAE face? And you’ve named a number of them there. I would put those under the category of sort of social consequences, I guess. Would you like to speak to what complications kids face?
Fiona Wardman 23:36
Yes. So, you know, as I mentioned before, with schooling and exams, there’s that stress, so kids first day of school, stress, right? All of those things can bring on an attack. It won’t necessarily happen every single time, but it can happen, and it usually does.
Trying to manage that is not, you know, I think it’s easier said than done without the right medications, but it’s not being able to go to social functions, not being able to go to school, not being able to take part in certain things because they may have an attack, or it might bring on an attack. Or they are having an attack, so they need to sit out.
That sort of isolates children. And there’s many other diseases and situations where that’s the case, and we have seen over the years, we’ve held some mental health workshops with adults and children to help them overcome that give them some tools and tips and tricks to use when they find themselves in that position.
But what we don’t want is to bring about any further bullying because a child can’t sit for an exam, can’t go to a birthday party, can’t go to school camp, is missing too much school, all of those things, and the stress, obviously, is a trigger.
So, what you’re doing is you’re just rolling over from one thing to the next, and the poor child is missing out. And, you know, it’s, it’s not great. It’s not great for them, for their self-esteem. It’s not great for getting through life and being what they want to be.
Megan Gilmour 25:19
Yes, and we’ll get more of that coming through as we talk about school. You know, there’s the illness, the disease consequences, there’s treatment consequences, and then there’s the social consequences.
And it just hearing that really, you know, squeezes the heart, doesn’t it, just to think that you could be in anticipation of some really positive things, and then here we are again, that’s not happening.
Fiona Wardman 25:54
Right
Megan Gilmour 25:54
That has knock on effects. And as you said, that could happen over and over again, and the triggers for that could be in a vicious cycle, exactly.
Fiona Wardman 26:09
Exactly.
Megan Gilmour 26:09
And I want to also just mention here again girls, and especially around those teenage years and I guess for boys as well. You know, those years where hormones are surging and changing and moving into adolescence and then into early adulthood, that would be quite a complex time, and we’ll come back to that a bit later.
So how are siblings and peers of kids with HAE impacted. And what should we know about that, or what should families – schools be across?
Fiona Wardman 26:47
Yeah, that’s an interesting one, I think, because we have seen in the past that a child within a family that has HAE is sort of treated with ‘kid gloves’, more so than the other. And there may be, you know, a little bit more – not connection, but rallying around that child to make sure they’re okay, and then the others feel left out.
I think it’s really important to educate the siblings on this disease, help them understand, so that they’re not impacted themselves negatively by the one that does have HAE.
And just going to the parents as well, we have found that parents are – the one that does have HAE – is so guilty for passing this disease on to their child, and especially through the years where we didn’t have any medication. Now we do, and it’s for pediatrics as well, we have medications available.
So, parents have that guilt and rallying around that child a bit more, and the others are a little bit left out. And you know, we have to make sure that we normalise this disease, and this can be normalised, although it’s rare and its life threatening, because there is availability of medications.
We are incredibly lucky in this disease to have so many available treatment options – on demand or acute – and also for preventative therapy and across the board. We’re incredibly lucky. So, any child really can have a normal life with those medications. And the only thing that would be different to their siblings would be needing to take their medication.
Megan Gilmour 28:45
Yes, that’s a really good sum up there, and it gives me a couple of flow on questions. Do you think that the medication, the availability of good medication, and the advances in that have to do with the fact that medical science has pinpointed the gene and has pinpointed the protein? And so, whilst it’s an invisible illness, medically there’s a really clear pathway of why it’s happening. And so, the therapies are targeted at that?
Because I’ve also had some interviews with diseases that aren’t getting the medication, aren’t getting the huge numbers of people associated with those. And so, I’m just wondering if that’s why there’s clarity around it?
Fiona Wardman 29:40
Interestingly, the first preventative or acute therapy was basically replacing the protein in our blood. So that’s still available. But the advances in this disease state are sort of further up the chain, so rather than just replacing, we’re stopping further up the chain, the ‘where it’s not working ‘right, and so we’re sort of turning the tap off to not allow the swelling to happen in, you know, very general terms.
And then all the way to the gene editing, which is another incredible situation for our disease as well, but you’ve got everything in between. And we have some of those medications available for stopping it further up the chain, in needle form, whether it’s a subcutaneous injection or it’s an IV injection, we now are getting those in tablet form, much easier for kids to take.
A lot of people are needle phobic, and when you do inject, sometimes you do get redness and a bit of stinging around the where you the injection site. But with a tablet, it’s so much easier. You don’t have to worry about cold chain, all of that stuff is taken away, and you pop a couple of pills, and your swelling stops, your attack stops, basically.
So, it’s the advancements from where it started to now is not the same thing rehashed. It’s basically more science figuring out where it’s going wrong, and then trying to stop that further up the chain in our bodies, to make sure that it doesn’t even get to the point where the blood vessels leak.
Megan Gilmour 31:31
Yes, great explanation for that. That’s so helpful, and it’s easy to see that an intervention up the chain and then when that intervention turns into a tablet form rather than injection form. Now we’re really talking about quality of life and reducing the burden or the impact of treatment, all those good things.
Fiona Wardman 32:01
Yes, can I just, sorry, add in there that not all of those things are available here yet. There are a couple of clinical trials going on the injections we’ve got, but there are the tablets are being trialed at the moment, and with great success. And there’s a couple of different companies that are doing that, and so they’re not available, but they’re hopefully coming and also available through the government that will cover the cost.
Megan Gilmour 32:27
Excellent. Thanks for putting that in. Another question that comes to me in listening to your great capture of effects on siblings, and also some dynamics in family, guilt, and, you know, parental attention and these sorts of things. In terms of the siblings, even kids with HAE you mentioned mental health is, is anxiety and other mental health issues in the picture here?
Fiona Wardman 33:04
Yes, absolutely. Because you have the anxiety around the next attack or the attack that you’re currently having. How long is it going to take all of those things. Depression – I suffered through some of that, before I had my diagnosis, before I had access to treatments, through, you know, ‘why me – why is this happening to me? What did I do?’
And then when you feel so sick and awful when an attack is coming on, and sometimes it can just come on and it’s slow, and sometimes it comes on and it’s fast, and you’re vomiting and you’ve got hot and cold sweats and you’re fainting and all of those sorts of things, and you’re lying there and you’re thinking ‘why? why is this happening to me?’ when you don’t have that diagnosis. Even when you do and you don’t have access, because we do have some criteria around access to preventative therapy, which we as an organisation are trying to overcome.
But in terms of people that do have access to it or don’t, there is still the thought that, yes, there’s depression about having the disease, there’s anxiety about the next attack, there is stress for what’s going to happen, and all of that, again, is just the vicious cycle of bringing on another attack. And you know when and when you’re not feeling well it’ll just keep happening.
Megan Gilmour 34:33
Yes, and I imagine that even for siblings there, there’s also anxiety around an attack happening and how that might affect the family
Fiona Wardman 34:44
Yes, absolutely, exactly, you’re about to go on holidays, or you’re about to have a birthday party and Christmas, or whatever it happens to be, and there’s so much excitement, and then one child is ill, and the others are missing out.
So it’s really important to make sure that, I mean, I can’t tell parents how to parent their children, but having that understanding, and you know, if there is this disease, making sure that it’s being monitored but also looked at, and making sure that the child is having the least amount of attacks as possible, so that the whole family can enjoy all the things that they want to do together.
Megan Gilmour 35:29
And siblings might even feel like – if they’ve been associated with a situation where an attack occurs – that they’re somehow responsible too, or feel worried for their sibling, that worried about the throat swelling and all of that sort of stuff. So, I think that’s worth-
Fiona Wardman 35:49
Yes, and you can’t stop kids from being kids, right? And we shouldn’t do that in this case either. Something we like to promote is that kids are doing sport, contact sport, or whatever they want to do. Let’s not stop the kids from doing what they want to because of the fear of an attack. The attack can be managed, as long as they have the diagnosis right, and they can get to a hospital if they don’t have access to preventative therapy.
Every patient with HAE in Australia should have access to on demand therapy – that’s children from two years of age and up. So, there is really no excuse not to be able to get on and do it. And if you do have a bump, or you do start with an attack somewhere, injection will stop it within sort of 15 minutes, and it’ll start to go down. So, there is the opportunity to live a normal life, to be a kid, to do all of the things you want to do, and then have taken the medication if you need it.
Megan Gilmour 36:50
A bit like an EpiPen, I guess,
Fiona Wardman 36:54
A bit like that. But that’s where we also want to stay away from the allergy analogy, right? Because it’s not an allergy. But yes, these, there’s more modern medications coming, which are more like an EpiPen in the way it’s injected. So, you know, they’re making all of these new ways of taking the medication, whether it’s on demand or for prophylaxis or preventative therapy easier for everybody.
Megan Gilmour 37:25
Excellent and thank goodness your lived experience has taken you into being an advocate. It’s just so wonderful that people like you are out there doing this work that has incredible benefits for people in circumstances of HAE.
Okay, so what’s the current population of school aged children with HAE? And how accurate is this – is the data tracked for example?
Fiona Wardman 37:58
No, the data is not really tracked. It’s, as I mentioned before, there’s around 100 kids that could potentially have HAE at this point, and we know about 30 or 40 of them.
So, I think we’re in in contact with a lot of the families. We do have people referred to us through their physicians for contact, so that we can help them and give them resources and different things that we have available. And because we do have the biggest sort of cohort of patients, whether that’s children or adults we find that the doctors come to us if they’re doing a clinical trial or whatever, so that we can get the word out.
So it’s important that everybody’s in contact with us, but to make sure that everybody is sort of counted, if I can use that as a term, because when we do go to the government to advocate for funded treatments, we need to know the numbers, so making sure that we have everybody counted and we, send in consumer comments or so forth when something is up for review, these sorts of things are important to get the information out to all of our patients and caregivers to be included.
Megan Gilmour 39:26
Yeah, that’s really important. And in terms of data, let’s just pop in there, and we don’t need to go back on it, because we’ve talked about it. But the fact that there could be students out there – indeed adults, but we’re here to talk about students – who are assumed to have an allergic reaction when they actually have HAE and may be lacking that diagnosis.
So, I think the number that you’re talking about is a base it’s a base number, right? It’s not capturing the undiagnosed people, children in what is a complex diagnosis. If GPs or doctors don’t understand that, well, not even that they don’t understand but they’re not aware of HAE, being a rare disease.
Fiona Wardman 40:19
Yes. I think the diagnosis all the time to diagnosis was 10 years to get the diagnosis, it’s come down a little bit.
And I have to say that we work with the Clinical Immunology Society of Australasia, and they do a lot of work to help immunologists and allergists understand this disease, and also it’s in the curriculum for general practitioners, as well as under the primary immune deficiency module, so that there is an offshoot to HAE, so that at least people have heard about it.
And I can say from some of my interactions with physicians, more and more students have heard of it, which is great. This is what we need, the new generation coming through do know about it, and can think about it when somebody does present with the symptoms.
Megan Gilmour 41:11
That’s excellent. So, do kids with HAE currently miss a lot of schools, and if so, why?
Fiona Wardman 41:18
Yes, so they can do, obviously, if they’re not diagnosed, and all of the stresses and the bits and pieces that we talked about that can bring on an attack.
If they’re not diagnosed, they can potentially have one or two or three attacks a month, which will keep them off school, depending on how severe the attack is. And then if you put that over 12 months of school, they’re missing a lot, and important exams and important social functions.
If the child is diagnosed and has doesn’t have access to treatment, I would suggest that preventative therapy is something that they need to consider and speaking to the immunologist and making sure that they do follow up, track the symptoms that they’re having, the attacks, making sure that they keep a diary. Our organisation has an electronic diary that you have on your phone that can log all of that to make sure that when you go to your doctor, you can show them how many attacks they’ve had, which will then help them get access to the treatments.
And of course, the next basket is the patients that aren’t diagnosed. And it could be, you know, a lot of kids.
But I think I forgot to mention the ones that do have access to treatments. And you know, sometimes the preventative therapies work, and sometimes there’s a shortcoming, which has certainly happened to me, and to make sure that those patients do have their on demand therapy, or talk to the doctor about upping the dose, or taking it more regularly, or whatever it happens to be, just to overcome that sort of short term need for good coverage of the preventative medicine.
Megan Gilmour 43:10
And all of that’s going to help with school.
Fiona Wardman 43:13
Absolutely, because you’re not missing it, you’re not having to worry about the next attack. You’re not having to worry about there’s an exam coming up. If all of that is taken care of before we get to that point, the kids can sail through and not have to worry about that. All they need to worry about is passing their exams.
Megan Gilmour 43:30
Yeah, which speaks to the advances that we’re seeing in medical science at the same time as more acknowledgement of rare diseases and the increase in chronic illness, we’re also seeing at the same time those lifesaving or quality of life inducing treatments. And I think this is why school matters so much, apart from the fact that education is so critical in a person’s life, so life changing,
Fiona Wardman 44:08
It sets you up, right, for the rest of your life, and the friends you make and the interactions you have and the social activity and the sporting involvement. All of those things set you up for the rest of your life. So, for those formative years, we want to make sure that the kids are well controlled, well managed, have access to whatever they need, and can live their life to the fullest with their friends.
Megan Gilmour 44:35
Yes. And so, this is the interaction – the positive interaction that we need to establish between health and education so that children can build a life worth living, and that they don’t become the identity of their disease and miss out on critical education, social connections, but also developmental milestones and shared community experiences that are formative.
So this is also why we are advocating for learn from anywhere at times when children can’t be in school, or if it’s not safe to be in school at any particular point in time in the management or process of their illness, disease or injury, and that means they can continue with two way digital connection so they’re not missing out on those vital moments if more school is missed.
And you and I have covered here the fact that we can talk generally about symptoms, side effects, consequences, but every child is also individual, even within the same disease category, their actual presentation, as you said, can be quite different, even in a single family.
So, what are the critical times in a kid with HAE’s school life.
Fiona Wardman 46:02
Oh, it would be, as I’ve mentioned, first day of school; moving schools; anything that is a positive experience, the really like or something they’re looking forward to; sport; exams; hormonal changes; all of those things. Or feeling, you know, being bullied and being depressed from that, even aside from this disease, all of that brings on negativity to the child and then puts them into a lower head space.
So, making sure that that’s recognised, and they’re talked with, I’m not going to say talk to, because they should be spoken with and about what’s going on. To make sure that there’s positivity and understanding around them, on what is going on, and acceptance as well, because part of this is accepting that you do have a rare disease, but also that you don’t have to put up with it if you don’t want to, because there are opportunities for medication and not having to worry about it.
But it’s basically right through from the day one when they go to school, go through the school gates, until when they leave and beyond, it’s one of those things all through their life. And everybody deals with different things differently. Some of us manage stress better than others. And, you know, there is not a one size fits all to this disease, and it really is about managing your own symptoms and your own way of dealing with the things that you know bring on these attacks.
Megan Gilmour 47:52
Yes, and then what should families and schools be prepared for at different times then? So, you’ve mentioned a few things. It’d be great to just kind of wrap those together.
Fiona Wardman 48:02
I really think education within the school, whether there’s a school nurse or the principal or the direct teacher or teachers there’s some information about the disease. On our website, we do have downloadable resources for that purpose, also letters for teachers that they can share, and the understanding of the disease, so that they aren’t left off to the side if they’re doing a running race or whatever it is, that if the child is having an attack, they don’t need to go home, they can stay at school.
Somebody can inject. They can learn how to self-inject, so that they’re not normal for 10 minutes rather than not normal for a whole day. So, it’s, you know, bringing some just trying to find the right word here, some normalcy I suppose, about the disease, so that the child can just get on with it.
And so, education of everybody that’s close to that child, even from preschool or even daycare and after school care, all of those people really need to know about this disease. So yes, if it’s a throat attack, that child, even if you’re giving them the medication, needs to go to hospital, just in case the throat does close over.
So, there’s, you know, some really important things they need to know. But if it’s just a hand attack, and when I say just a hand attack, it’s still very painful, but it doesn’t necessarily mean they have to go home, they can have the medication and feel better. And the earlier that we use the on-demand therapy, the better it works, so not delaying those sorts of things, but just learn about the disease and how to manage it.
Megan Gilmour 49:51
Yes, great wrap up. And what that brings forward for me is something, and I haven’t put it this way before, but I will. So, that early intervention, using the best advancements in medical science are being implemented and used, so there’s a quality of life there immediately if possible, or there’s mitigation of symptoms, and there’s of course safety at the forefront of all of that.
And so, our point of view is we should be doing that for school as well. If we can intervene that way with drugs and medication, we can intervene with the right things in schools, so that all the benefits of medical treatment and advancements and interventions can enable what needs to happen at school to take place.
So, at this point in the conversation, I typically remind listeners viewers about the Disability Standards for Education, which is a national regulation under the Disability Discrimination Act. And the DSE applies to every single school in Australia, which makes it a fantastic instrument for these students we’re talking about, and schools know exactly what’s required of them when they associate an illness, a medical condition, a disease, with a particular student.
It means that we’re reducing inequality through the standards to enable that student with HAE to have the same opportunities as their peers who don’t have HAE. It means access to the classroom – so for us that includes when the student is not in the classroom, so it might be at home.
And it also means access to the curriculum, because you’re having that connection to school, learning alongside peers, and being able to do that. Additional supports at school if it’s needed, and in this case, I’m hearing that there may be need for staff to be trained to support medications, but also to understand, as you said, the disease and how the school can implement different supports to enable that equality of opportunity.
It also addresses stigma and victimisation harassment. You’ve mentioned bullying a lot on this call, and so this is one that really stands out for me here, where you know schools are sensitive to the fact that students with HAE might be in that situation with their peers and creating a school environment where, as you’ve said, and your organisation can assist in providing that vital information to de-stigmatise.
So, we just placed that there. It’s a fantastic regulation and includes an individual education plan in order for the accommodations to be to be put in place, and those accommodations would depend on the student and their particular circumstances, as you’ve highlighted, and what’s going on for them at different times in their school journey – times that might be more stressful, and people being aware of that and having accommodations and adjustments in place to address those.
So, if exam time causes issues, then how can we reduce the stress at exam times? Do we have a different room? Do we provide a longer period of time in order to just to support. So, they’re just a few things that come to mind.
So, what are the biggest challenges or vulnerabilities – you’ve covered a lot of these – is there anything you’d like to add to this question – at school, we’re talking about. What are the biggest challenges or vulnerabilities facing kids with HAE at school?
Fiona Wardman 54:27
There’s no more that I can think of off the top of my head, but just reiterating that the child is normal for most of the time, except when they’re having a painful attack. So, letting them do what they want to do, how they want to do it, and just accommodating, as you were just talking about if they are sick because of having an attack at the lead up to exam time, then they may need a little bit of extra time.
But not singling them out. It’s really about keeping the kids happy as well, to do what they want to do, not stopping them from doing anything, and the important thing to know is that they can do that with access, even if they don’t have preventative therapy, they can with on demand therapy. So, these kids can do whatever they like and love, that shouldn’t have a problem.
Megan Gilmour 55:29
Yes, I love that. That comes back to identity as well, and it also takes us back full circle to that moment of equality of opportunity, so meaning that our environments adjust and accommodate, so that these students have equal opportunities to their peers, and they’re not singled out or specifically identified in a way that stigmatises.
Fiona Wardman 55:55
Exactly.
Megan Gilmour 55:56
So, I think that’s really an important thing that the standards can add weight to.
Fiona Wardman 56:03
I mean, kids go through a lot anyway, right, through school, they’ve got so many other things to worry about than, than having a rare disease and then being singled out for that. It’s a lot more complicated than what it used to be, I think. And I don’t think I would want to be a child in this, in these times, going through what these kids are at the moment.
However, we can help them in whatever way, shape or form, that is to not single them out, not to shine a torch light on them, to say, hey, this person’s got something wrong with them. It just eases everything, it just makes everything so much happier and easier, so that kids can just get on with being kids and learning and playing and being social like they should, and getting into sport and whatever else it is that they love doing,
Megan Gilmour 56:55
Yes, and getting up to a bit of mischief if they want to as well [laughter].
Fiona Wardman 56:57
Exactly, exactly.
Megan Gilmour 56:59
Yes, this is, these are real key points here. Life has so much already, and as we know and we remember from the playground and school, just navigating through all of your developmental stages and life itself has enough to offer in terms of complexity, in terms of, interacting with life.
And so, the ways that we can intervene early for school, and obviously the very critical treatment for disease, the earlier the better. And the reduction of stigma and the reduction of difference, without taking away the positive differences and diversity that each child brings and enabling them to be themselves and to be able to bring themselves to their places, that the community is also super important, right?
Fiona Wardman 57:17
Let’s not hold them back
Megan Gilmour 57:18
Yes. It’s not what we it’s not necessarily us helping them. It’s what they contribute to us as well. And so, this is something I’d like to leave there.
We’ve talked about vulnerabilities, challenges, we talked about medications at school and normalcy. Do you think, or I’ll put it another way, what’s happening now in schools, from your point of view, to address these challenges and vulnerabilities that we’ve talked about, and is it enough? Is there something else you would like to see happening?
Fiona Wardman 58:45
I think, just more of the same, really, and making sure that parents are also active in the education role for their children, to enlighten the people that they are with at school, and obviously their friends’ parents and so forth, to help them understand that the disease is what it is.
It’s manageable with medication, and it’s, yes it is life threatening, but things can be done. The child can just get on with it. Education is key, and I said it before, knowledge is power, and if everybody understands what is expected or what is necessary, and if you need information, reach out to us.
At our website there are downloadable resources there, there’s videos, there’s all sorts of things to help with the education and awareness around this disease, to help people understand exactly what it’s like. And, yes, when you see somebody that’s got a massively swollen face, it looks horrendous, but it’s swelling, it will go away. But it doesn’t necessarily change the person. It is accepting what it is, and it will go away, and we can just keep moving forward.
Megan Gilmour 1:00:17
Is there anything else we have missed on school quality of life for HAE?
Fiona Wardman 1:00:24
I think just precautions, and it sort of feeds back to what happens at school when there is an attack. But let’s not stop kids from going to school camps. Let’s make sure that somebody is educated on giving the child the medication. Let’s not hold them back.
You know, we want kids to get to have that interaction, to get those life experiences and do all of those fun things. And it’s up to us as adults to make sure that the people around our children are educated and aware of what may happen, what will happen, and what has happened, and how we can overcome that.
Megan Gilmour 1:01:11
Fantastic Fiona, what’s your call to action for hereditary angioedema in seven words or less?
Fiona Wardman 1:01:20
All patients can live a normal life.
Megan Gilmour 1:01:24
Love that. A big shout out to Fiona and to HAE Australasia. You can find them at haeaustralasia.org.au [https://www.haeaustralasia.org.au]. Head there for resources, all the good things that Fiona has talked about, information, connections and give generously, because we need organisations like this doing the work that Fiona is doing with colleagues and making sure that that call to action can become a reality.
Thank you for joining me today, Fiona.
Fiona Wardman 1:02:12
Thank you so much for having me on the podcast.
Megan Gilmour 1:03:03
It’s our absolute pleasure.
Thanks everyone for tuning in. Help us turn up the volume so all kids are seen and heard. Follow along, like share comment or leave us a review, because we love hearing from you. And if you want to donate, head to MissingSchool.org.au [https://www.missingschool.org.au], because every dollar makes a difference.
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